Members Login
Medical NewsEPG Search
Frequent Searches:
Browse Other Languages
Spanish Drug Data (España)
German Drug Data (Deutsch)
Norwegian Drug Data (Norge)
Netherlands Drug Data (Nederlands)
Swedish Drug Data (Sverige)
German Drug Data (Deutsch)
Norwegian Drug Data (Norge)
Netherlands Drug Data (Nederlands)
Swedish Drug Data (Sverige)
YouTube - Featured Content
Want to submit or suggest content for the epgonline.org YouTube Clinical channel?
Contact us here
epgonline.org is not responsible for content on any 3rd party website
Are you a Doctor or Healthcare Professional? Authorise Viewing
Please register to access disease diagnosis, patient management, physician tools.
By viewing the content of this web page you are both confirming your status as a healthcare professional and agreeing to our terms of use.
Change language
Current language database:
United Kingdom
Medicine information : FABRAZYME 5 mg Solution for Infusion
| Drug class description : | Alimentary tract and metabolism products – enzymes |
| Generic Name : | Agalsidase Beta |
| Drug description : | Powder for concentrate for solution for infusion White to off-white lyophilized cake or powder |
| Presentation : | Each vial of Fabrazyme contains a nominal value of 5 mg of agalsidase beta. After reconstitution with 1.1 ml water for injections, each vial of Fabrazyme contains 5 mg/ml of agalsidase beta. The reconstituted solution must be diluted further. Agalsidase beta is a recombinant form of human a-galactosidase A and is produced by recombinant DNA technology using a mammalian Chinese Hamster Ovary (CHO) cell culture. The amino acid sequence of the recombinant form, as well as the nucleotide sequence which encoded it, are identical to the natural form of a-galactosidase. |
| Indications : | Fabrazyme is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease (a-galactosidase A deficiency). |
| Adult Dosage : | Fabrazyme treatment should be supervised by a physician experienced in the management of patients with Fabry Disease or other inherited metabolic diseases. The recommended dose of Fabrazyme is 1mg/kg body weight administered once every 2 weeks as an intravenous infusion. The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions. No dose adjustment is necessary for patients with renal insufficiency. Studies in patients with hepatic insufficiency have not been performed. |
| Child Dosage : | There is no experience in children. The safety and efficacy of Fabrazyme in children below the age of 16 years have not been established and no dosage regimen can presently be recommended in these patients. |
| Elderly Dosage : | There is no experience in children. The safety and efficacy of Fabrazyme in patients older than 65 years have not been established and no dosage regimen can presently be recommended in these patients. |
| Contra Indications : | Life threatening hypersensitivity (anaphylactic reaction) to the active substance or any of the excipients. |
| Special Precautions : | Almost all patients are expected to develop IgG antibodies to agalsidase beta using the advised starting dose, mostly within 3 months of initiation of treatment. After up to 2 years of therapy, 63% of patients who were antibody positive show a significant reduction in antibody titer over time. The remaining 37% of patients demonstrated a plateau in their antibody titer. In a limited number of patients IgE antibodies were demonstrated. Two of these patients were subsequently rechallenged uneventfully with Fabrazyme and continue to receive treatment. Patients with antibodies to agalsidase beta have a higher risk of infusion-associated reactions defined as any related adverse event occurring during the infusion or until the end of the infusion day (See Adverse Reactions). These patients should be treated with caution when re-administering agalsidase beta. Initially, approximately half of the patients experienced infusion-associated reactions (See Adverse Reactions). In the phase 3 clinical study and its extension, infusion-associated reactions were managed by slowing the rate of infusion and by pre-treating the patients with antihistamines, paracetamol, ibuprofen and/or corticosteroids. As with any intravenous protein product, allergic-type hypersensitivity reactions are possible. Patients experiencing mild or moderate hypersensitivity reactions when treated with agalsidase beta during clinical trials have continued therapy after a reduction in the rate of infusion (~0.15 mg/min; 10 mg/hr) and pre-treatment with antihistamines, paracetamol, ibuprofen and/or corticosteroids. A small number of patients has experienced reactions suggestive of immediate (Type I) hypersensitivity. If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of the administration of Fabrazyme should be considered and an appropriate treatment has to be initiated. The current medical standards for emergency treatment are to be observed. The effect of Fabrazyme treatment on the kidneys may be limited in patients with advanced renal disease. Studies have not been performed to assess the potential effects of Fabrazyme on impairment of fertility. |
| Interactions : | No interaction studies and no in vitro metabolism studies have been performed. Based on its metabolism, agalsidase beta is an unlikely candidate for cytochrome P450 mediated drug-drug interactions. Fabrazyme should not be administered with chloroquine, amiodarone, benoquin or gentamycin due to a theoretical risk of inhibition of intra-cellular a-galactosidase activity. Pregnancy and lactation: There are no adequate data from the use of agalsidase beta in pregnant women. Animal studies do not indicate direct or indirect harmful effects with respect to embryonal/foetal development. Fabrazyme should not be used during pregnancy unless clearly necessary. Agalsidase beta may be excreted in milk. Because there are no data available on effects in neonates exposed to agalsidase beta via breast milk, it is recommended to stop breast-feeding when Fabrazyme is used. |
| Adverse Reactions : | Adverse drug reactions (ADRs) reported to be related to Fabrazyme administered at a dose of 1mg/kg in a total of 58 patients treated up to 12 months are listed by system organ class and frequency (very common:> 10%, common: 5-10%) in the table below. ADRs were mostly mild to moderate in severity: Nervous system disorders Very common: Headache, tremor Common: Paraesthesia, dizziness, somnolence Eye disorders : Common: Vision abnormal, lacrimation abnormal Cardiac disorders : Common: Tachycardia, bradycardia Vascular disorders : Very common: Hypertension, Common: Flushing, pallor. Respiratory, thoracic and mediastinal disorders Very common Rhinitis, dyspnoea, Common Bronchospasm, throat tightness. Gastrointestinal disorders : Very common: Nausea, vomiting Common: Abdominal pain. Skin and subcutaneous tissue disorders : Common: Pruritus. Musculoskeletal and connective tissue disorders : Very common: Myalgia, pain at the extremities Common: Back pain, leg pain. Renal and urinary disorders : Common: Albuminuria. General disorders and administration site conditions : Very common: Rigors, temperature changed sensation, fever, oedema of the extremities Common: Chest pain, fatigue, , pain, asthenia, malaise Initially, approximately half of the patients experienced infusion-associated reactions. These reactions consisted most often of fever/chills. Additional symptoms included mild to moderate dyspnoea, throat tightness, chest tightness, flushing, pruritus, urticaria, face oedema, angioneurotic oedema, rhinitis, bronchial constriction, tachypnea and/or wheezing, moderate hypertension, hypotension, tachycardia, palpitations, abdominal pain, nausea, vomiting, infusion-related pain including pain at the extremities, myalgia, and headache. The infusion-associated reactions were managed by a reduction in the rate of infusion together with the administration of non-steroidal anti-inflammatory medicinal products, antihistamines and/or corticosteroids. After up to 2 years less than 37% of the patients experienced infusion-associated reactions. The majority of these reactions can be attributed to the formation of IgG antibodies and/or complement activation. In a limited number of patients IgE antibodies were demonstrated. Two of these patients were subsequently rechallenged uneventfully with Fabrazyme and continue to receive treatment. |
| Manufacturer : | Genzyme Therapeutics |
| Drug Availability : | (POM) |
Free registration to access disease diagnosis, patient management, physician tools.
Only registered users have access to the full EPG Online Knowledge Base including drug search tools, interactive disease Knowledge Centres via the EPG Knowledge Library - with clinical and evidence based physician and research tools, MiMedia (build your own clinical media collection with everything from key opinion leader essays to mode of action videos and other multi-media content), the EPG Survey panel, health news updates and much much more.
Already Registered?
Please log-in now using our member login box.
Not a member?
Don't worry, registration is quick and FREE! We welcome all Healthcare professionals, doctors, nurses and medical students.
Register today to have full access to a wealth of drug data, educational and evidence based interactive guides across all major therapeutic areas, disease management, and clinical tools.
As a practicing Healthcare professional, you can also opt-in to join our market research panel – www.epgsurvey.com – and get paid for sharing your expert clinical opinions!
REGISTER today it only takes a minute! and it's FREE
Having problems?
Use our forgotten password facility or email us at: contact@epgonline.org